非糖尿病成人患者低血糖的鉴别诊断是复杂的,包括多种疾病,包括由功能性β细胞疾病引起的内源性高胰岛素血症。后者也被指定为nesidioblasy或非胰岛素瘤胰腺源性低血糖综合征(NIPHS)。临床上,这种罕见的疾病表现为非特异性的肾上腺素能和神经糖量减少症状,因此,经常被忽视。结合仔细的临床评估,口服葡萄糖耐量试验,禁食72小时,断层和功能成像,和侵入性胰岛素测量可以导致正确的诊断。由于缺乏对病情的病理生理理解,保守治疗方案有限,且大多无效.因此,目前,几乎所有患者都接受了部分或整个胰腺的手术切除。因此,除了更快的诊断,需要更复杂、侵入性较小的治疗方案来缓解患者的危险和破坏性症状。根据我们部门一名23岁男子患有这种疾病的病例,我们对有关这种情况的医学文献进行了广泛的回顾,并在此对这种有趣的疾病进行了全面的讨论,包括从流行病学到治疗的所有方面。
Differential diagnosis of hypoglycemia in the non-diabetic adult patient is complex and comprises various diseases, including endogenous hyperinsulinism caused by functional β-cell disorders. The latter is also designated as
nesidioblastosis or non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS). Clinically, this rare disease presents with unspecific adrenergic and neuroglycopenic symptoms and is, therefore, often overlooked. A combination of careful clinical assessment, oral glucose tolerance testing, 72 h fasting, sectional and functional imaging, and invasive insulin measurements can lead to the correct diagnosis. Due to a lack of a pathophysiological understanding of the condition, conservative treatment options are limited and mostly ineffective. Therefore, nearly all patients currently undergo surgical resection of parts or the entire pancreas. Consequently, apart from faster diagnosis, more elaborate and less invasive treatment options are needed to relieve the patients from the dangerous and devastating symptoms. Based on a case of a 23-year-old man presenting with this disease in our department, we performed an extensive
review of the medical literature dealing with this condition and herein presented a comprehensive discussion of this interesting disease, including all aspects from epidemiology to therapy.